However, a more recent Italian population-based study suggests that the incidence may be closer to 10%, or perhaps close to 5%. On the severe end of the spectrum, frontotemporal dementia (FTD), particularly the behavioral variant of FTD (bvFTD), marked by severe apathy and progressive declines in socially appropriate behavior, judgment, and self-control, as well as personality change, has been reported to range from 5% to 27% in various series.

The most common deficits are in executive function (demonstrated by difficulties with emotional and impulse control, flexible thinking, self-monitoring, planning and prioritizing, organization, task initiation, and working memory), verbal fluency, and social cognition (demonstrated by difficulties interpreting the emotional states of others and lack of insight in social situations). Several neuropsychological domains may be affected. It has been reported that upwards of 45% of individuals with ALS have some degree of cognitive impairment at some time during their illness. White matter structural abnormalities in the frontal and temporal lobes of individuals with ALS who do not demonstrate evidence of cognitive decline have also been identified. Recent studies of both the genetics and neuropathology of ALS have reinforced the understanding that while the syndrome of ALS by definition involves the motor system, wider frontotemporal degeneration may give rise to at least some degree of cognitive and behavioral dysfunction. Various subtypes of ALS have been identified: A diagnostic feature of ALS, typically not seen in other neurodegenerative disorders, is the presence of hyperreflexia in segmental regions of muscle atrophy, unaccompanied by sensory disturbance.Īt presentation, limb involvement occurs more often than bulbar involvement. Other findings may include muscle fasciculations, muscle cramps, and lability of affect, but not necessarily mood.

LMN signs in ALS include weakness, muscle wasting (atrophy), hyporeflexia, muscle cramps, and fasciculations.Įarly manifestations may vary, with affected individuals most often presenting with either asymmetric focal weakness of the extremities (stumbling or poor handgrip) or bulbar findings (dysarthria, dysphagia). LMNs, located in the brain stem and spinal cord, innervate striated muscle. UMN signs in ALS include hyperreflexia, extensor plantar response, and increased muscle tone. Additional UMN influences on the LMN are carried over descending pathways of the brain stem. Upper motor neurons (UMNs), located in the motor cortex of the frontal lobe, send their axons through the great corticofugal tracts to the brain stem (corticobulbar neurons) and the spinal cord (corticospinal neurons) to influence patterned activity of the lower motor neurons (LMNs). And by introducing a striking dark wood Art Deco bookcase or low-profile mid-century modern design by Paul McCobb into your living room, your signed first editions won’t be the only items making a statement.Motor symptoms occur as the result of degeneration of both upper and lower motor neurons. If you don’t wish to fill your storage piece so that your collection is snug within its confines, incorporate extra space to allow for additional displays and decorative objects. Even those who don’t covet the perfect home library can benefit from an attractive display case, as bookcases can easily double as charming étagères.Ĭontemporary and customizable options make it easier for you to find the perfect bookcase for your style and stacks. Whether you proudly shelve your books in regal mahogany or behind glass cabinet doors, a bookcase - or perhaps more than one - is essential to creating a cozy nook for any book lover. Americans of means during the 19th century took to amassing art as well as rare books, and antique bookcases of the era, rife with hand-carved decorative accents and architectural motifs, were ideal for displaying their handsome leather-bound wares.Īlthough our favorite titles may change over the years, the functionality and beauty of their home within our home is timeless. And, of course, as with any treasured objects, a book lover’s volumes were suddenly worthy of a luxurious display - enter the bookcase. When auction houses and book dealers proliferated by the late 17th century, the bibliophile was born.

As long as curious people have collected stories, we have needed a place to stow them away and preserve them.

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